PASH Syndrome in Iran


Sora Yasri 1 , * , Viroj Wiwanitkit 2

1 KMT Primary Care Center, Bangkok, Thailand

2 Dr. D. Y. Patil University, Pune, India

How to Cite: Yasri S, Wiwanitkit V. PASH Syndrome in Iran. J Skin Stem Cell.6(1):e98423. doi: 10.5812/jssc.98423.


Journal of Skin and Stem Cell: 6 (1); e98423
Published Online: December 25, 2019
Article Type: Letter
Received: September 27, 2019
Revised: November 12, 2019
Accepted: December 18, 2019

Dear Editor,

We read a paper entitled “PASH syndrome: The first case report from Iran,” by Mansouri et al. with a great interest (1). Mansouri et al. mentioned that “PASH syndrome despite rarity should be considered as a possible cause of complex skin manifestations” (1).

PASH syndrome is an uncommon problem. It is a clinical syndrome that consists of pyoderma gangrenosum, acne, and hidradenitis suppurativa (2). Cutaneous inflammation is the main pathology of PASH. The underlying pathogenesis of this syndrome is over-activation of the innate immune system that results in the increased production of the interleukin (IL)-1 family (2). Genetic heterogenicity is reported in PASH syndrome (3). The NCSTN gene is reported to have an association with PASH syndrome (4). The PASH syndrome is sporadically reported from several countries around the world, such as the USA, Spain, China, etc. (5-7). A novel multimodal treatment with infliximab, cyclosporine, and dapsone is recommended for PASH treatment (8). The present report by Mansouri et al. (1) is not the first case report from Iran. Faraji Zonooz et al. reported a similar case from Iran (3). The genetic variation in that case is well described (data available at



  • 1.

    Mansouri P, Naraghi Z, Hesami Z, Hajiha N, Chalangari Kasir A, Azizian Z. PASH syndrome: The first case report from Iran. J Skin Stem Cell. 2019;5(4). e86096. doi: 10.5812/jssc.86096.

  • 2.

    Cugno M, Borghi A, Marzano AV. PAPA, PASH and PAPASH syndromes: Pathophysiology, presentation and treatment. Am J Clin Dermatol. 2017;18(4):555-62. doi: 10.1007/s40257-017-0265-1. [PubMed: 28236224].

  • 3.

    Sonbol H, Duchatelet S, Miskinyte S, Bonsang B, Hovnanian A, Misery L. PASH syndrome (pyoderma gangrenosum, acne and hidradenitis suppurativa): A disease with genetic heterogeneity. Br J Dermatol. 2018;178(1):e17-8. doi: 10.1111/bjd.15740. [PubMed: 28626985].

  • 4.

    Faraji Zonooz M, Sabbagh-Kermani F, Fattahi Z, Fadaee M, Akbari MR, Amiri R, et al. Whole genome linkage analysis followed by whole exome sequencing identifies nicastrin (NCSTN) as a causative gene in a multiplex family with gamma-secretase spectrum of autoinflammatory skin phenotypes. J Invest Dermatol. 2016;136(6):1283-6. doi: 10.1016/j.jid.2016.02.801. [PubMed: 26968259].

  • 5.

    Gracia-Cazana T, Frias M, Rosello R, Vera-Alvarez J, Gilaberte Y. PASH syndrome associated with osteopoikilosis. Int J Dermatol. 2015;54(9):e369-71. doi: 10.1111/ijd.12827. [PubMed: 26175185].

  • 6.

    Niv D, Ramirez JA, Fivenson DP. Pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH) syndrome with recurrent vasculitis. JAAD Case Rep. 2017;3(1):70-3. doi: 10.1016/j.jdcr.2016.11.006. [PubMed: 28203623]. [PubMed Central: PMC5294749].

  • 7.

    Li C, Xu H, Wang B. Is SAPHO syndrome linked to PASH syndrome and Hidradenitis suppurativa by Nicastrin mutation? A case report. J Rheumatol. 2018;45(11):1605-7. doi: 10.3899/jrheum.171007. [PubMed: 30068763].

  • 8.

    Staub J, Pfannschmidt N, Strohal R, Braun-Falco M, Lohse P, Goerdt S, et al. Successful treatment of PASH syndrome with infliximab, cyclosporine and dapsone. J Eur Acad Dermatol Venereol. 2015;29(11):2243-7. doi: 10.1111/jdv.12765. [PubMed: 25352307].

  • Copyright © 2019, Journal of Skin and Stem Cell. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License ( which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited.